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Systemic AAV9 gene transfer in adult GM1 gangliosidosis mice reduces lysosomal storage in CNS and extends lifespan

GM1 gangliosidosis (GM1) is an autosomal recessive lysosomal storage disease where GLB1 gene mutations result in a reduction or absence of lysosomal acid β-galactosidase (βgal) activity. βgal deficiency leads to accumulation of GM1-ganglioside in the central nervous system (CNS). GM1 is characterize...

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Bibliografiske detaljer
Udgivet i:Hum Mol Genet
Main Authors: Weismann, Cara M., Ferreira, Jennifer, Keeler, Allison M., Su, Qin, Qui, Linghua, Shaffer, Scott A., Xu, Zuoshang, Gao, Guangping, Sena-Esteves, Miguel
Format: Artigo
Sprog:Inglês
Udgivet: Oxford University Press 2015
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC4492398/
https://ncbi.nlm.nih.gov/pubmed/25964428
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddv168
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